[A description of early hydrocephalus with macrocephaly, cerebral palsy, epilepsy, intellectual disability, and growth retardation by Norwegian novelist Amalie Skram (1846-1905)]. / Hans (Hansemann) Tønnesen, en diagnostisk gåte fra Amalie Skram.

In her novel «S.G. Myre¼ written in 1890, Norwegian author Amalie Skram describes Hans (Hansemann) Tønnesen, a 16-year-old boy with an enormous head, convulsions, and a rudimentary command of language. He is bullied by children in the street and is held in low esteem by his mother, who refers to him as a beast, crazy, or «a cross¼. Presumably, the boy has hydrocephalus with macrocephaly, epilepsy, and mental retardation. His retarded growth, small teeth and cracked skin suggest growth hormone deficiency resulting from his hydrocephalus. A couple of interesting details, such as his «splaying his fingers and moving them in various directions to make himself understood¼ and his «becoming red with exertion and grimacing terribly¼ when trying to speak, suggest extrapyramidal, dyskinetic cerebral palsy. He may also be deaf. A cousin of Skram's shared several of these traits and is a likely model for Hansemann. A question that occurs to a modern reader is to what degree Hansemann is mentally retarded, or whether he is wrongly perceived as such because of his physical challenges and his communication problems.